Download bilateral Hypoglossal nerve Palsy Due to breast Cancer Skull base

Case Report
Bilateral Hypoglossal Nerve Palsy Due
to Breast Cancer Skull Base Metastases
Jennifer M. Matro, MD, Rohit Walia, MD, Kathryn Tumelty, CRNP, Tara Morrison, MD, and Lori J. Goldstein, MD
Abstract
Despite advances in endocrine therapy and
chemotherapy, metastatic disease ultimately develops
in about 30% of women diagnosed with early-stage
breast cancer. Critical locations of bone metastases
may result in specific symptoms. Metastases to the
calvarium have the potential to cause cranial nerve
palsies. While there have been isolated case reports of
unilateral cranial nerve XII palsy, bilateral hypoglossal
nerve palsy has never been reported. We report the
first known case of bilateral hypoglossal nerve palsy
in a 49-year-old woman with estrogen receptor–
positive/progesterone receptor–positive, HER2/
neu-negative metastatic breast cancer. The patient
underwent palliative radiation therapy with near
complete resolution of symptoms. When a patient
with metastatic cancer develops unusual neurological
symptoms, a high index of suspicion for skull base
disease must be maintained, and local therapy can
improve quality of life.
Introduction
Despite advances in endocrine therapy and chemotherapy,
metastatic disease ultimately develops in about 30% of women
diagnosed with early-stage breast cancer.1 The most common
sites of metastasis, beyond regional lymph nodes, are bone,
lungs, liver, and brain.2 Critical locations of bone metastases
may result in specific symptoms. Metastases to the calvarium
have the potential to cause cranial nerve palsies. The most
common cranial nerves affected in metastatic breast cancer are
V and VII, although there have been isolated case reports of
unilateral cranial nerve XII palsy. We report the first known
case of bilateral hypoglossal nerve palsy in a 49-year-old woman
with estrogen receptor–positive/progesterone receptor–positive
(ER+/PR+), HER2/neu-negative metastatic breast cancer.
fuse bone metastases 2 years prior. She had not responded to
first-line hormonal therapy with tamoxifen and zoledronic acid,
and so was switched to albumin-bound paclitaxel and bevacizumab with zoledronic acid. Bevacizumab was discontinued due
to proteinuria and hypertension 4 months prior to her current
presentation, at which time she was receiving only albuminbound paclitaxel and zoledronic acid.
The patient had developed the sensation of a swollen tongue
approximately 1 week prior and was treated with fluconazole,
dexamethasone, and nystatin for presumed thrush. She returned with persistent tongue swelling and dysarthria. She
denied dysphagia. On examination, the uvula was midline,
and the tongue was flaccid and not edematous. Gag reflex was
intact. Cranial nerves II through XI were intact, but the patient
was unable to protrude and laterally move her tongue in either
direction, indicating bilateral hypoglossal nerve palsy. The
remainder of her neurological exam was normal. The patient
was admitted to the hospital for expedited workup and management.
figure 1. MRI T2 Axial Image Showing Metastatic
Disease Involving the Bilateral Basiocciput
Case Report
A 49-year-old woman with stage IV, pT4 pN1 cM1, ER+/PR+,
HER2/neu-negative breast cancer presented with subacute onset
of a swollen tongue and difficulty speaking. The patient had
been diagnosed with de novo stage IV breast cancer with dif-
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www.ajho.com Hypoglossal canals
november 2014
Bil ateral Hypoglossal Nerve Palsy Due to Breast Cancer Skull Base Metastases
Practical Application
• Management of patients with metastatic cancer requires a multidisciplinary team approach.
• Clinicians must maintain a high index of suspicion in patients with
metastatic cancer that atypical symptoms may be related to malignancy.
• Selection of the optimal imaging study will aid in diagnosis.
• Recognition of abnormal cranial nerve examination findings [AUTHORS: SHOULD MAKE COMPLETE SENTENCE TO BE PARALLEL
WITH OTHERS.]
The patient had been taking prochloperazine for chemotherapy-induced nausea. To rule out a dystonic reaction to
compazine, benztropine was administered without relief of
symptoms. An MRI of the brain with and without gadolinium
subsequently revealed extensive calvarial metastases involving
the skull base, including the clivus, bilateral occipital condyles,
and C1 vertebra. Tumor was thought to be compressing the hypoglossal nerves as they traversed the hypoglossal canals at the
skull base bilaterally. Figure 1 includes a T2 axial image showing metastatic disease involving the left and right basiocciput.
Figure 2 shows a coronal post-contrast view of the hypoglossal
canals, and Figure 3 demonstrates the skull base metastases on
bone scan.
The patient underwent neurosurgical evaluation to ensure
stability of the C1/C2 junction and superior cervical spine,
which was intact. She subsequently received radiation therapy
to the clivus, C1 vertebral body, and adjacent skull base. Prescribed dosage was 3000 cGy in 10 fractions delivered with opposed lateral fields with 6 MV photons. On follow-up approximately 1 month later, the patient reported improved speech
and was able to eat all foods. On examination, she was able to
protrude the tongue but with persistent deviation to the right.
Unfortunately, the patient had rapidly progressive disease, most
markedly in the liver and mediastinum. Two weeks after followup, she developed a malignant pericardial effusion and expired
due to multisystem organ failure.
Discussion
Metastatic disease is found in 5% of women at the time of
breast cancer diagnosis. For those women with early-stage breast
cancer, about 30% will ultimately develop distant metastases.1
Cranial nerve palsies are rare complications of metastatic cancer, occurring in less than 10% of patients with brain metastases.2 However, cranial nerves may be compromised due to bone
metastases without intracranial lesions. Metastases to the skull
base, which occurs in 4% of patients with cancer, with breast
cancer being the most common primary site,3 may cause compression of and damage to cranial nerves as they exit the skull
through the basal foramina. The most frequently affected cranial nerves in women with metastatic breast cancer are V and
VII, with 70% and 60% of patients, respectively, having involve-
VOL. 10, NO. 5
ment of these nerves.4 Several English-language case reports
have been published describing unilateral hypoglossal nerve
palsy due to skull base metastasis, but no reports have documented bilateral involvement.4-8 In the case series by Hall et al,4
only 4 of 10 patients had involvement of cranial nerve XII, 1
with isolated hypoglossal palsy and all with unilateral involvement. Greenberg et al8 performed a retrospective analysis of 43
patients with metastases to the skull base. Nine of 43 patients
had involvement of the occipital condyle, and all 9 patients
had unilateral 12th nerve palsy. Metastases at the basilar part of
the occipital bone (basiocciput) and bilateral occipital condyles
were likely the cause of our patient’s symptoms.
figure 2. Coronal Post-Contrast Image Showing
Skull Base Metastases Involving the Bilateral
Hypoglossal Canals
Jugular tubercles
Hypoglossal canals
Our case represents the first report of bilateral hypoglossal
nerve palsy due to skull base bone metastases. The diagnosis
was made by MRI imaging, the imaging study of choice for
skull base involvement, although bone scan with SPECT could
be used for this purpose.9 While the average survival time for
patients with cranial nerve palsy due to skull base metastasis
can be up to 2 years, those with intracranial metastases have a
median survival ranging from 5.6 to 14.4 months, depending
on number of lesions, performance status, hormone and HER2
receptor status, and response to systemic treatment.10,11 Despite
a significant clinical improvement following local radiotherapy,
our patient ultimately died from her cancer due to systemic
progression 3 months after her symptoms developed.
This case indicates that when a patient with metastatic cancer
develops unusual neurological symptoms, a high index of suspicion for skull base disease must be maintained, especially in
THE AMERICAN JOURNAL OF HEMATOLOGY/ONCOLOGY
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Case Report
figure 3. Bone Scan Demonstrating Bilateral Skull
Base Metastases (white arrows)
RT. LAT
LT. LAT
Series Description: Wholebody
Radiopharmaceutical 1: 910.2 MBq (24.60
mCi) MDP
Energy Window Group 1: 99m Technetium
WB Technique: 1PS
Scan Velocity: 2.00 mm/sec
Table Traverse: 0.00 mm
Table Height: 0.00 mm
the absence of intracranial lesions. Aggressive local therapy can
lead to near-complete resolution of symptoms and improvement
in quality of life for patients with an otherwise significantly limited life expectancy.
5. Pavithran K, Doval DC, Hukku S, Jena A. Isolated hypoglossal nerve palsy due to skull base metastasis from breast cancer.
Australas Radiol. 2001;45:534-535.
6. Endo K, Okano R, Kuroda Y, et al. Renal cell carcinoma with
skull base metastasis preceded by paraneoplastic signs in a chronic hemodialysis patient. Int Med. 2001;40(9):924-930.
7. Shiraishi T, Yanagida H, Takada K, Yasuhara Y. Unusual cranial metastasis from hepatoma presenting as isolated unilateral
hypoglossal nerve paresis. Neurol Med Chir. 1992;32:166-168.
8. Greenberg HS, Deck MDF, Vikram B, et al. Metastasis to
the base of the skull: clinical findings in 43 patients. Neurology.
1981;31:530-537.
9. Fukumoto M, Osaki Y, Yoshida D, et al. Dual-isotope SPECT
diagnosis of a skull-base metastasis causing isolated unilateral hypoglossal nerve palsy. Ann Nuc Med. 1998;12(4):213-216.
10. Melisko ME, Moore DH, Sneed PK, et al. Brain metastases
in breast cancer: clinical and pathologic characteristics associated
with improvements in survival. J Neurooncol. 2008;88(3):359-365.
11. Lee SS, Ahn JH, Kim MK, et al. Brain metastases in breast
cancer: prognostic factors and management. Breast Cancer Res
Treat. 2008;111(3):523-530.
Affiliations: Jennifer M. Matro, MD, is from the Abramson
Cancer Center, University of Pennsylvania, Philadelphia; Rohit
Walia, MD, is from the Fox Chase Cancer Center, Department
of Diagnostic Imaging, Philadelphia, PA; and Kathryn Tumelty,
CRNP, Tara Morrison, MD, and Lori J. Goldstein, MD, are from
the Fox Chase Cancer Center, Department of Medical Oncology.
Disclosures: TK
Address correspondence to: Lori J. Goldstein, MD, 333 Cottman Avenue, Philadelphia, PA 19111; phone: 215-728-2985; fax:
215-728-2880; email: [email protected]
References
1. Howlader N, Noone AM, Krapcho M, et al. SEER Cancer Statistics Review, 1975-2008. National Cancer Institute. http://seer.
cancer.gov/csr/1975_2008/, based on November 2010 SEER
data submission, posted to the SEER web site, 2011. Accessed
September 19, 2014.
2. Carty NJ, Foggitt A, Hamilton CR, Royle GT, Taylor I. Patterns of clinical metastasis in breast cancer: an analysis of 100
patients. Eur J Surg Onc. 1995;21:607-608.
3. Laigle-Donadey F, Taillibert S, Martin-Duverneuil N, et al.
Skull-base metastases. J Neurooncol. 2005;75(1):63-69.
4. Hall SM, Buzdar AU, Blumenscheine GR. Cranial nerve palsies in metastatic breast cancer due to osseous metastasis without
intracranial involvement. Cancer. 1983;52:180-184.
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www.ajho.com november 2014